Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress

BACKGROUND: Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare sporadic disorder with an incidence of approximately 1 per 8 million live births. The phenotypic appearance consists of short stature, sculptured nose, alopecia, prominent scalp veins, small face, loss of subcutaneous fat...

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Detalles Bibliográficos
Autores principales: Paradisi, Mauro, McClintock, Dayle, Boguslavsky, Revekka L, Pedicelli, Christina, Worman, Howard J, Djabali, Karima
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2005
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1183198/
https://www.ncbi.nlm.nih.gov/pubmed/15982412
http://dx.doi.org/10.1186/1471-2121-6-27

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